Pathophysiological Concepts and Treatment of Camptocormia

Abstract

Camptocormia is a disabling pathological, non-fixed, forward bending of the trunk. The clinical definition using only the bending angle is insufficient; it should include the subjectively perceived inability to stand upright, occurrence of back pain, typical individual complaints, and need for walking aids and compensatory signs (e.g. back-swept wing sign). Due to the heterogeneous etiologies of camptocormia a broad diagnostic approach is necessary. Camptocormia is most frequently encountered in movement disorders (PD and dystonia) and muscles diseases (myositis and myopathy, mainly facio-scapulo-humeral muscular dystrophy (FSHD)). The main diagnostic aim is to discover the etiology by looking for signs of the underlying disease in the neurological examination, EMG, muscle MRI and possibly biopsy. PD and probably myositic camptocormia can be divided into an acute and a chronic stage according to the duration of camptocormia and the findings in the short time inversion recovery (STIR) and T1 sequences of paravertebral muscle MRI. There is no established treatment of camptocormia resulting from any etiology. Case series suggest that deep brain stimulation (DBS) of the subthalamic nucleus (STN-DBS) is effective in the acute but not the chronic stage of PD camptocormia. In chronic stages with degenerated muscles, treatment options are limited to orthoses, walking aids, physiotherapy and pain therapy. In acute myositic camptocormia an escalation strategy with different immunosuppressive drugs is recommended. In dystonic camptocormia, as in dystonia in general, case reports have shown botulinum toxin and DBS of the globus pallidus internus (GPi-DBS) to be effective. Camptocormia in connection with primary myopathies should be treated according to the underlying illness.

Clinical Definition of Camptocormia in Parkinson's Disease

“…Camptocormia is an involuntary, marked flexion of the thoracolumbar spine apparent during standing or walking and resolving in the supine position; it is mostly found in movement disorders, particularly Parkinson's disease (PD). [1][2][3] Although the clinical syndrome of camptocormia is universally accepted and meanwhile defined by characteristic myopathological 4 and radiological 5 changes of the paravertebral muscles, an unambiguous clinical definition of camptocormia is lacking. In some publications, a UPDRS III-item 28 ≥ 2 is used for definition, 6 but in most studies, camptocormia is defined on the basis of the angle of bending.…”

A new therapeutic strategy with istradefylline for postural deformities in Parkinson’s disease

“…However, withdrawal of the dopamine agonist may worsen the motor symptoms of PD. Other treatment options including deep brain stimulation and botulinum toxin injection are available, but they are unsatisfactory in some patients [2]. Istradefylline, an adenosine A2A receptor antagonist, is a novel non-dopaminergic drug available for the treatment of PD in Japan [3].…”

Treatment with istradefylline for postural abnormalities in Parkinson’s disease

“…L-dopa infusions improved camptocormia and antecollis in PD, especially when abnormal postures were OFF-state associated [23]. Surgical approaches include deep brain stimulation (of about 80 patients, about 60% showed improvement of posture); orthopedic spinal correction, and unilateral pallidotomy [8]. Lastly, physiotherapy can achieve improvements.…”

“…The etiology of postural abnormalities in PD remains poorly understood [4,5,8]. Camptocormia in PD was reported to worsen in the OFF state and thus suggested to arise due to rigidity.…”

“…Further etiologies of camptocormia include primary and secondary myopathy, proprioceptive dysregulation, dystonia, inflammation/myositis as well as functional and medicationinduced disorders. Additionally, an impaired perception of verticality is associated with postural abnormalities in PD [4,5,8].…”